Recurrence-Free Survival in Composite Hemangioendothelioma: A Case Study and Updated Systematic Review.

: Composite hemangioendothelioma (CHE) is a rare vascular endothelial tumor with borderline malignancy. This study presents a case of CHE and an updated systematic review of previously reported cases, providing insights into recurrence patterns and survival outcomes. : A comprehensive electronic search was conducted across PubMed, Scopus, the Cochrane Library, and Web of Science up to 31 December 2024, to identify eligible case reports. Kaplan-Meier curves were used to estimate event-free survival. : We report a 61-year-old man with a splenic lesion associated with weight loss and abdominal pain persisting for 1 year. Intraoperative findings revealed an enlarged spleen and multiple hepatic deposits. Splenectomy and liver biopsy revealed a well-demarcated, nodular tumor measuring 160 × 145 × 100 mm, with histological and immunohistochemical findings consistent with CHE, complicated by hepatic metastasis. Of 405 potentially eligible studies, 59 were included in the review, covering cases from 2000 to 2024, with a peak in 2020 and 2023. The median age of patients was 42 years, with the most common tumor sites being the lower extremities (30.48%), followed by the face, head, and neck (20.95%), and upper extremities (18.1%). Surgical intervention was the most common treatment (60.95%). Recurrence-free survival was observed in 42.86% of cases, while 15.24% experienced recurrence with or without metastasis. Two patients (1.90%) died from the disease. The median recurrence-free survival was 48 months (95% CI: 7.3-88.7). : CHE exhibits significant morphological variation and can mimic other vascular tumors. Accurate diagnosis is crucial for proper prognosis and avoiding overtreatment due to misdiagnosis as more aggressive neoplasms. Patients with high-risk CHE should undergo closer surveillance to ensure timely detection of progression.